Background

Idiopathic pleuroparenchymal fibroelastosis (iPPFE) is a rare interstitial lung disease characterized by unique radiological and pathological findings. However, pathological evaluations are available only in a limited number of patients. Therefore, several clinical diagnostic criteria have been proposed. Nevertheless, the applicability of these criteria has not yet been validated. Moreover, the clinical course of iPPFE and its prognosis have not yet been completely elucidated.

Methods

The present study assessed previously proposed clinical diagnostic criteria by comparing the clinical features between pathologically diagnosed iPPFE (p-iPPFE) and clinically diagnosed iPPFE (c-iPPFE). Subsequently, the clinical features of iPPFE were characterized and compared with those of idiopathic pulmonary fibrosis (IPF, n = 323).

Results

Clinical characteristics of c-iPPFE (n = 27) and p-iPPFE (n = 35) were similar. No significant difference was observed in terms of prognosis between c-iPPFE and p-iPPFE. The number of patients with iPPFE (both c-iPPFE and p-iPPFE) who developed lung cancer was significantly lower than that of patients with IPF. However, acute exacerbation (AE) showed similar incidence in patients with iPPFE and IPF. Survival of patients with iPPFE was significantly worse than that of patients with IPF (5-year survival rate: 38.5% vs. 63.5%, p < 0.0001), and the most common cause of death was chronic respiratory failure (73.8%), followed by AE (14.3%). Male gender was the only poor prognostic factor of iPPFE.

Conclusion

The present study demonstrated efficiency of clinical diagnosis and also revealed clinically important characteristics of iPPFE that should be considered for management of iPPFE.

中文翻译：

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与特发性肺纤维化相比，胸膜实质性纤维弹性病的病程和预后。

背景

特发性胸膜实质纤维弹性增生（iPPFE）是一种罕见的间质性肺疾病，其特征是独特的放射学和病理学发现。但是，病理评估仅在有限的患者中可用。因此，已经提出了几种临床诊断标准。但是，这些标准的适用性尚未得到验证。此外，iPPFE的临床病程及其预后尚未完全阐明。

方法

本研究通过比较病理诊断的iPPFE（p-iPPFE）和临床诊断的iPPFE（c-iPPFE）的临床特征，评估了先前提出的临床诊断标准。随后，对iPPFE的临床特征进行了表征，并与特发性肺纤维化（IPF，n = 323）进行了比较。

结果

c-iPPFE（n = 27）和p-iPPFE（n = 35）的临床特征相似。在c-iPPFE和p-iPPFE的预后方面未观察到显着差异。发生肺癌的iPPFE患者（c-iPPFE和p-iPPFE）均明显低于IPF患者。但是，iPPFE和IPF患者的急性加重（AE）发生率相似。iPPFE患者的生存率显着低于IPF患者（5年生存率：38.5％对63.5％，p <0.0001），最常见的死亡原因是慢性呼吸衰竭（73.8％），其次由AE（14.3％）。男性是iPPFE的唯一不良预后因素。

结论

本研究证明了临床诊断的效率，并且还揭示了iPPFE的临床重要特征，应考虑对其进行管理。